الفهرس 
Anesthetic Considerations in PorphyriaOnly 14 pages are availabe for public view
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Abstract
Inherited metabolic diseases, while individually rare
conditions, contribute significantly to pediatric morbidity and
mortality. Anesthetists may encounter patients with inherited
metabolic diseases presenting for both emergency and elective
surgery. The management of these patients is often challenging
due to the multisystemic manifestations of many IMDs.
Catastrophic metabolic decompensation may occur in the
perioperative period and a multidisciplinary approach is
essential to ensure safe management of these patients.
Anesthesia of hepatic porphyric patients can trigger
potentially serious complications even though documented
accidents are rare. Although most anesthetic agents have been
used in cases of latent Porphyria (except highly contraindicated
barbiturates), caution is necessary. If an acute attack
is suspected, only agents known to be safe in this context
should be used. This rarely occurring disorder must not be
underestimated. Any suspicion must lead to diagnosis
assessment and possibly to research into the family history.
Inherited defects in glycogen metabolism prevent the
mobilization of glucose from glycogen and typically cause the
accumulation of glycogen within tissues such as the liver or
muscles. The management of the GSD involves maintaining an
adequate blood glucose level and provision of alternate energy
sources.
Management of anesthesia in patients with disorders of
amino acid metabolism is directed toward maintenance of
intravascular fluid volume and acid-base homeostasis. Use of
anesthetics that could evoke seizures may be questionable in
view of the likely presence of seizure disorders in these
patients.